From Volume 48, Issue 5, May 2021 | Pages 409-416
Fibrous dysplasia is a benign condition characterized by replacement of bone by a fibro-osseous tissue. This article describes the aetiology, diagnosis and classification of the condition. We discuss the clinical presentation of fibrous dysplasia along with its craniofacial effects. The presentation of fibrous dysplasia in the dental setting is described, along with specific implications for dental and orthodontic management of these patients.
Fibrous dysplasia (FD) is a progressive, benign fibro-osseous condition characterized by replacement of normal bone by fibrous tissue and irregular bony trabeculae.1,2 Although the World Health Organization categorizes FD within various groups of diseases, the most descriptive of these is as an osteochondrodysplasia arising from a chromosomal abnormality.3 The actual incidence and prevalence are difficult to estimate as mild cases and asymptomatic lesions may go undiagnosed, but are reported to represent 1% of primary bone tumours, and approximately 5–7% of benign bone tumours.1,2,4 The majority of lesions are detected by the age of 30 years, with no gender predilection.
The aetiology of FD is linked to a mutation in the GNAS1 gene located at chromosome 20q13.2-13.3. The mutation develops sporadically during early pregnancy, and therefore, while FD is a genetic condition, it is not inherited, or passed on to offspring of affected individuals. The gene mutation leads to abnormal differentiation of osteoblasts, which are thought to contribute to the development of lesions through the formation of fibro-osseous tissue. Common presenting features are fractures, deformity and pain.
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